ENDOCRINE MANAGEMENT IN PATIENTS WITH PRADER-WILLI SYNDROME

Abstract

PRADER-WILLI SYNDROME IS A GENETIC DISORDER RESULTING FROM THE LOSS OF GENETIC EXPRESSION IN THE PATERNAL CHROMOSOME 15, CHARACTERIZED BY HYPOTONIA, MENTAL RETARDATION, BINGE EATING AND OBESITY LINKED TO HYPOTHALAMIC DYSFUNCTIONS. THE OBJECTIVE OF THIS STUDY WAS TO EVALUATE THE METABOLIC ALTERATIONS OF CHILDREN WITH PRADER-WILLI SYNDROME FROM TWO CLINICAL CASES COLLECTED FROM APAE IN THE CITY OF ANÁPOLIS. THE CHARACTERISTICS FOUND WERE CORRELATED WITH INFORMATION FROM ARTICLES IN THE PUBMED AND SCIELO DATABASE. THEREFORE, IT IS OF FUNDAMENTAL IMPORTANCE TO ELABORATE A STRATEGY OF HUMANIZED, HOLISTIC AND OBJECTIVE TREATMENT ACCORDING TO THE METABOLIC ASPECTS OF THE PATIENTS IN SEARCH OF AN EFFECTIVE BIOPSYCHOSOCIAL DEVELOPMENT AND AN IMPROVEMENT OF THE QUALITY OF LIFE OF THESE PATIENTS.